Sex Steroid Priming for Induction of Puberty in Thalassemia Patients with Pulsatile Reversible Hypogonadotrophic Hypogonadism

Chatterjee, Ratna; Mukhopadhyay, Tarak Nath; Chandra, Sharmila; Bajoria, Rekha

doi:10.3109/03630269.2011.630121

Cited by 9 publications

(6 citation statements)

References 10 publications

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“…Chatterjee et al 2011 [39] have since confirmed feasibility for low dose sex steroid priming in their Indian cohort as 80% reached pubertal maturation which was most effective in younger patients with minimal iron overload.…”

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confidence: 99%

A Review of Endocrine Disorders in Thalassaemia

De¹,

Mistry²,

Wright³

et al. 2014

OJEMD

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Endocrine dysfunction in thalassaemia is amongst the most common complication and is principally attributed to excessive iron overload and suboptimal chelation. The prevalence is quite high particularly in multiethnic populations but determining the prevalence is often difficult due to the widespread heterogeneity of the population and timing of exposure to chelation therapy. Disturbances in growth, pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes and disorderly bone growth are commonly encountered. Early detection and institution of appropriate transfusion regimen and chelation therapy and treatment of complications are the keys to managing this population including regular follow. In this article, we review the literature in relation to the various endocrine complications encountered in thalassaemia.

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confidence: 99%

A Review of Endocrine Disorders in Thalassaemia

De¹,

Mistry²,

Wright³

et al. 2014

OJEMD

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“…In CHH, a non‐response to LDSS is expected. In HC, pituitary siderosis is likely the main factor for DP and is often an irreversible condition, 43 although spontaneous recovery of the hypothalamus–pituitary–gonadal (HPG) function after a sex steroid priming has been also reported 66 …”

Section: Pathogenesis and Diagnostic Work‐upmentioning

confidence: 99%

Iron overload disorders: Growth and gonadal dysfunction in childhood and adolescence

Tenuta,

Cangiano,

Rastrelli

et al. 2024

Pediatric Blood & Cancer

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Hemochromatosis (HC) is characterized by the progressive accumulation of iron in the body, resulting in organ damage. Endocrine complications are particularly common, especially when the condition manifests in childhood or adolescence, when HC can adversely affect linear growth or pubertal development, with significant repercussions on quality of life even into adulthood. Therefore, a timely and accurate diagnosis of these disorders is mandatory, but sometimes complex for hematologists without endocrinological support. This is a narrative review focused on puberty and growth disorders during infancy and adolescence aiming to offer guidance for diagnosis, treatment, and proper follow‐up. Additionally, it aims to highlight gaps in the existing literature and emphasizes the importance of collaboration among specialists, which is essential in the era of precision medicine.

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“…The evidence that we have at the moment on females, however, is based mostly on β-Thalassemic patients, who are affected in 60-80% of the cases by hypogonadotropic hypogonadism: In a 6-year prospective study concerning children (boys and girls) with absent or arrested puberty and β-Thalassemia, sex hormones priming proved to be successful in inducing puberty in 80% of the subjects, suggesting that the estradiol priming could be as useful as the testosterone priming in the awakening of the hypothalamus-pituitary axis in the CDGP female patients 60 .…”

Section: Primingmentioning

confidence: 99%

Pubertal delay: the challenge of a timely differential diagnosis between congenital hypogonadotropic hypogonadism and constitutional delay of growth and puberty

et al. 2020

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Distinguishing between Constitutional Delay of Growth and Puberty (CDGP) and CongenitalHypogonadotropic Hypogonadism (CHH) may be challenging. CDGP and CHH appear to belong to the same clinical spectrum (with low sex hormones and low LH and FSH), although one is classically transient and known as a self-limited form of delayed puberty (CDGP) while the other is permanent (CHH). Thus, the clinical history and the outcomes of these two conditions require different approaches, and an adequate and timely management for the patients is mandatory. Since the initial presentation of CDGP and CHH is almost identical and given the similarities of CDGP and partial forms of CHH (i.e. patients with partial and early interrupted pubertal development) the scientific community has been struggling to find some diagnostic tests able to allow an accurate differential diagnosis between these two conditions in delayed puberty. In this review we provide an up to date insight on the tests available, their meanings and accuracy, as well as some clues to effectively differentiate between constitutional pubertal delay and pathologic CHH.

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Sex Steroid Priming for Induction of Puberty in Thalassemia Patients with Pulsatile Reversible Hypogonadotrophic Hypogonadism

Cited by 9 publications

References 10 publications

A Review of Endocrine Disorders in Thalassaemia

A Review of Endocrine Disorders in Thalassaemia

Iron overload disorders: Growth and gonadal dysfunction in childhood and adolescence

Pubertal delay: the challenge of a timely differential diagnosis between congenital hypogonadotropic hypogonadism and constitutional delay of growth and puberty

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